Long Term Remission of Capillary Leak Syndrome Associated with Monoclonal Gammopathy with Progression to Multiple Myeloma After Autologous Stem Cell Transplantation: a Case Report and Review of the Literature.

BACKGROUND: Clarkson's disease is a very rare entity characterised by acute episodes of systemic oedema and severe hypotension associated with paraproteinaemia. Its classical treatment relies on methylxanthine combined with terbutaline. Although this prophylactic therapy reduces the mortality rate, relapses are frequent. Eighty percent of patients with Clarkson's disease present with monoclonal gammopathy of unknown significance (MGUS). The risk of progression to multiple myeloma is 1% per year. CASE DESCRIPTION: Here, we present a 49-year-old woman who suffered multiple such episodes requiring treatment in the intensive care unit. Treatment with terbutaline and theophylline was ineffective. She was diagnosed with multiple myeloma (MM) 8 years after the first of these acute episodes. Antimyeloma treatment with bortezomib and dexamethasone was started, followed by autologous haemopoietic transplantation, with no further acute episodes since then. CONCLUSION: Our case is, to our knowledge, unique because eradication of MM was followed by complete disappearance of acute episodes of capillary leakage. Our case report is also the first to support the use of bortezomib and dexamethasone in this setting. Furthermore, autologous peripheral blood progenitor cell transplantation consolidated the MM stringent complete remission achieving a very long progression-free survival (> 11 years) of both MM and Clarkson's disease.

Influence of antiplatelet-anticoagulant drugs on the need of blood components transfusion after vesical transurethral resection.

AIMS: The effect of the antithrombotic preventive therapy on haemorrhage keeps uncertain. We investigate the influence of the antiplatelet and anticoagulant drugs (AP/AC drugs) on the transfusion requirement after vesical transurethral resection (VTUR). We also describe the epidemiology of the blood components transfusion in our department. MATERIALS AND METHODS: Retrospective observational study of a series of patients needing blood transfusion at the Urology Department between June 2010 and June 2013. Selection of 100 consecutive patients who were transfused after VTUR due to bladder transitional cell carcinoma (BTCC) (group A = GA). CONTROL GROUP: 100 consecutive patients who underwent VTUR due to BTCC and were not transfused (group B = GB). Transfusion criteria: Haemoglobin < 8 g/dl + anaemia symptoms. Age, gender, associated AP/AC treatment, secondary diagnoses, toxics, tumour stage and grade were analysed. RESULTS: 212 patients required transfusion of a blood component. 169 were men (79%) and 43 women (21%). Median age 77.59 years (SD 9.42, range 50-92). Secondary diagnoses: Diabetes Mellitus 64%, high blood pressure 77%, dyslipidemia 52%. 60% of patients were previously treated with AP/AC drugs. Average Haemoglobin pre-transfusion values: 7.4 g/dl (DE ± 0.7). Average Haemoglobin post-transfusion values: 8.9 g/Dl (DE ± 0.72). Most frequent transfusion indications were bladder cancer (37%), kidney cancer (11%), prostate cancer (8%), benign prostatic hyperplasia (BHP) (8%), other urological diagnoses (36%). Intraoperative transfusions indicated by the anaesthesiologist: kidney cancer (33%), BPH (28%). Patients who underwent VTUR due to BTCC were older in GA (77.59 years SD 9.42) than in GB (68.98 years SD 11.78) (p = 0.0001). Similar gender distribution (15 women in GA and 24 in GB). Less patients were asked to keep their treatment with ASA 100mg (AcetylSalicylicAcid) in GA (25.64%) than in GB (50%) (p = 0.0330). More aggressive tumour grade in GA (p = 0.0003) and higher stage in GA (p = 0.0018) regardless of concomitant treatment with AP/AC drugs. CONCLUSIONS: The pathologies which most needed blood components' transfusions in the Urology Department were (in order of frequency): bladder cancer, kidney cancer, prostate cancer, prostate adenoma. ASA100mg did not influence the transfusion's requirements in VTUR due to BTCC. Tumour stage and higher grade have a greater influence in transfusion's requirements than concomitant AP/AC treatment. The heterogeneity of AP/AC protocols does not allow to establish the benefit of stopping those drugs before surgery in terms of avoiding blood transfusions when performing a VTUR.

Prognostic impact of pre-transplantation computed tomography and 67gallium scanning in chemosensitive diffuse large B cell lymphoma patients undergoing hematopoietic stem-cell transplantation.

OBJECTIVE: In the present study, we evaluated computed tomography (CT) and (67)gallium scanning ((67)Ga scan) pre-transplant as prognostic factors for overall survival (OS) and event-free survival (EFS) in patients with diffuse large B cell lymphoma, undergoing high-dose chemotherapy and stem-cell transplantation. PATIENTS AND METHODS: Forty-two patients were included. Of these, 9 (21%) had both positive CT and (67)Ga scans, 17 (41%) negative results with both techniques, and 16 (38%) positive CT/negative (67)Ga scan. Whole-body planar imaging and single-photon emission computed tomography (SPECT) were performed 72 h after an intravenous administration of (67)Ga citrate measuring between 7 mCi and 10 mCi (259-370 MBq). RESULTS: Patients with positive CT/positive (67)Ga scan had a significantly worse EFS and OS at 5 years than those with negative (67)Ga scan regardless of whether it was associated with a positive or a negative CT scan (29% and 16% vs. 81% and 93% vs. 88% and 100%, respectively, P < 0.001). After a median follow-up of 43 months (range 4-130 months), no differences were observed between patients with negative CT/negative (67)Ga scan and those with positive CT/negative (67)Ga scan, with an EFS and OS at 5 years of 88% versus 81% and 100% versus 93%, respectively. In multivariate analysis, the presence of a pre-transplant positive CT/(67)Ga scans adversely influenced both EFS and OS [HR 8, 95% confidence interval (CI) (1.4-38), P = 0.03 and HR 2; 95% CI (1.3-8), P = 0.02, respectively]. CONCLUSIONS: (67)Ga scan helps to identify, in the pre-transplant evaluation, two groups with a different outcome: one group of patients with positive CT and negative (67)Ga scans pre-transplant, who showed a favorable outcome with a low rate of relapse, and the other group of patients with both positive CT and (67)Ga scans pre-transplant, who showed a poor prognosis and did not benefit from autologous stem-cell transplantation. They should have been offered other therapeutic strategies.

Immunophenotyping of plasma cells in multiple myeloma.

Multiparametric immunophenotyping of multiple myeloma (MM) and other plasma cell (PC) dyscrasias represents an attractive approach not only for research purposes but also in clinical practice. Based on well-established antigenic patterns, discrimination between myelomatous and normal PCs can be easily achieved in various types of samples, and this can be particularly valuable for the differential diagnosis between MGUS and MM and for monitoring residual disease in the latter. In addition, immunophenotyping may be an alternative and more reproducible method than morphology for evaluating PC infiltration, as well as for specifically analyzing DNA content and the cell-cycle distribution of different subsets of PCs. Despite the widespread use, standardization of methods and protocols still remains a challenge. In this chapter, we describe in detail the protocols and precise instructions for specimen collection, sample preparation, together with the methods for staining PCs and flow cytometry, data acquisition, and data analysis, including the more recent developments in the field. We highlight the most frequent limitations, and provide troubleshooting and practical recommendations that could help to solve them. The goal of this chapter is to emphasize the relevance of methodological issues in order to obtain reproducible and high-quality results regarding the phenotypic analysis of PCs.